article · FACE
Alveolar soft part sarcoma is an uncommon soft-tissue malignancy representing approximately one per cent of all sarcomas. It primarily arises in adolescents and young adults, displaying distinct histological traits, variable clinical behaviour, and a persistent lifelong risk of recurrence. Recognition of its clinical and imaging characteristics is vital to enable prompt detection and appropriate therapeutic intervention. A clinical report outlines this disease in an eleven-year-old girl who presented with right upward dystopia and proptosis caused by a highly vascularised mass. The lesion was identified as an alveolar soft part sarcoma. Treatment followed a multidisciplinary pathway involving pre-operative embolisation to manage the vascular tumour, followed by surgical resection and adjuvant radiotherapy to target residual disease.
Because alveolar soft part sarcoma is exceptionally rare and carries a lifelong threat of recurrence, clinical awareness is essential. Outlining specific paediatric presentations, such as orbital tumours causing abnormal eye positioning, helps medical practitioners recognise atypical features earlier. This awareness guides timely diagnosis and supports the deployment of combined treatment regimens involving embolisation, surgery, and radiotherapy to improve disease management.
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Alveolar soft part sarcoma (ASPS) is a rare soft-tissue tumor exhibiting characteristic histological features and variable clinical behavior with a lifelong risk of recurrence. It accounts for 1% of all sarcomas and mostly presents in adolescents and young adults. Awareness of clinical and radiological features may help in early diagnosis and proper management. We report a case of an 11-year-old girl presenting with right upward dystopia and proptosis caused by a highly vascular mass, which was revealed to be an ASPS. Management included pre-operative embolization, surgical resection and adjuvant radiotherapy.
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DOI: 10.1177/27325016261487582
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